Idiopathic retroperitoneal fibrosis, including IgG4-related disease


Definition

  • Retroperitoneal fibrosis encompasses a spectrum of inflammatory and fibrosing diseases involving the retroperitoneal structures.

  • Etiology not clearly understood in 2/3 cases; hence, idiopathic retroperitoneal fibrosis (IRF)

  • Some studies argue that IRF is part of the spectrum of IgG4-related disease.

Clinical features

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